syndrom delece 22q11 [22q11 Deletion Syndrome]

tematický
2
Perzistentní odkaz   https://www.medvik.cz/link/D058165
Definice

Stav s proměnlivou konstelaci fenotypů z důvodu delece polymorfismu v místě chromozomu 22q11. Mezi postižení způsobených delecí na chromozomu 22q11 patří několik syndromů, např. DiGeorgeův syndrom, velokardiofaciální syndrom a syndrom konotrunkálních a obličejových anomálií. S tímto syndromem jsou spojeny další variabilní vývojové problémy a schizoidní vlastnosti. Ne všechny delece 22q11 způsobují syndrom delece 22q11.

Condition with a variable constellation of phenotypes due to deletion polymorphisms at chromosome location 22q11. It encompasses several syndromes with overlapping abnormalities including the DIGEORGE SYNDROME, VELOCARDIOFACIAL SYNDROME, and CONOTRUNCAL AMOMALY FACE SYNDROME. In addition, variable developmental problems and schizoid features are also associated with this syndrome. (From BMC Med Genet. 2009 Feb 25;10:16) Not all deletions at 22q11 result in the 22q11deletion syndrome.

DUI
D058165 MeSH Prohlížeč
CUI
M0537456
Předchozí užití
Chromosome Deletion (1997-2010); Chromosomes, Human, Pair 22 (1997-2010)
Historická pozn.
2011
Veřejná pozn.
2011

C Nemoci
C05.660.207.103 syndrom delece 22q11 2
C05.660.207.103.500 DiGeorgeův syndrom 43
C05.660.207.219 Costellův syndrom 3
C05.660.207.240 kraniosynostózy 56
C05.660.207.325 Donohueův syndrom 1
C05.660.207.410 holoprosencefalie 9
C05.660.207.532 Loeysův-Dietzův syndrom 7
C05.660.207.536 megalencefalie 7
C05.660.207.620 mikrocefalie 78
C05.660.207.690 Noonanové syndrom 52
C05.660.207.707 plagiocefalie 3
C05.660.207.720 platybazie 12
C14.240.400 vrozené srdeční vady 1 563
C14.240.400.021 syndrom delece 22q11 2
C14.240.400.021.500 DiGeorgeův syndrom 43
C14.240.400.044 Alagillův syndrom 19
C14.240.400.090 koarktace aorty 203
C14.240.400.172 Barthův syndrom 6
C14.240.400.200 cor triatriatum 3
C14.240.400.210 anomálie koronárních cév 119
C14.240.400.220 crisscross srdce 1
C14.240.400.280 dextrokardie 21
C14.240.400.340 otevřená tepenná dučej 108
C14.240.400.395 Ebsteinova anomálie 46
C14.240.400.422 ectopia cordis 1
C14.240.400.450 Eisenmengerův syndrom 23
C14.240.400.560 defekty srdečního septa 164
C14.240.400.592 heterotaxe 7
C14.240.400.701 levokardie 2
C14.240.400.725 Marfanův syndrom 105
C14.240.400.787 Noonanové syndrom 52
C14.240.400.849 Fallotova tetralogie 163
C14.240.400.915 transpozice velkých cév 109
C14.240.400.920 trikuspidální atrézie 11
C14.240.400.960 Fallotova trilogie 2
C14.240.400.970 Patauův syndrom 6
C14.240.400.975 syndrom trizomie 18 5
C14.240.400.980 Turnerův syndrom 272
C14.240.400.990 jednokomorové srdce 3
C14.280 nemoci srdce 2 874
C14.280.400 vrozené srdeční vady 1 563
C14.280.400.044 syndrom delece 22q11 2
C14.280.400.044.500 DiGeorgeův syndrom 43
C14.280.400.090 koarktace aorty 203
C14.280.400.172 Barthův syndrom 6
C14.280.400.200 cor triatriatum 3
C14.280.400.210 anomálie koronárních cév 119
C14.280.400.220 crisscross srdce 1
C14.280.400.280 dextrokardie 21
C14.280.400.340 otevřená tepenná dučej 108
C14.280.400.395 Ebsteinova anomálie 46
C14.280.400.450 Eisenmengerův syndrom 23
C14.280.400.560 defekty srdečního septa 164
C14.280.400.592 heterotaxe 7
C14.280.400.701 levokardie 2
C14.280.400.725 Marfanův syndrom 105
C14.280.400.787 Noonanové syndrom 52
C14.280.400.849 Fallotova tetralogie 163
C14.280.400.915 transpozice velkých cév 109
C14.280.400.920 trikuspidální atrézie 11
C14.280.400.960 Fallotova trilogie 2
C14.280.400.970 Patauův syndrom 6
C14.280.400.975 syndrom trizomie 18 5
C14.280.400.980 Turnerův syndrom 272
C14.280.400.990 jednokomorové srdce 3
C15.604.451.249 syndrom delece 22q11 2
C15.604.451.249.500 DiGeorgeův syndrom 43
C16.131 vrozené vady 1 753
C16.131.077.019 syndrom delece 22q11 2
C16.131.077.019.500 DiGeorgeův syndrom 43
C16.131.077.065 Alagillův syndrom 19
C16.131.077.095 Angelmanův syndrom 35
C16.131.077.121 Barthův syndrom 6
C16.131.077.137 Bloomův syndrom 11
C16.131.077.229 Carneyův komplex 8
C16.131.077.245 ciliopatie 9
C16.131.077.250 Cockayneův syndrom 4
C16.131.077.256 Costellův syndrom 3
C16.131.077.272 de Langeové syndrom 4
C16.131.077.299 hluchoslepota 65
C16.131.077.313 Donohueův syndrom 1
C16.131.077.327 Downův syndrom 510
C16.131.077.350 ektodermální dysplazie 33
C16.131.077.371 Fraserův syndrom 2
C16.131.077.393 Gardnerův syndrom 21
C16.131.077.401 heterotaxe 7
C16.131.077.410 holoprosencefalie 9
C16.131.077.445 incontinentia pigmenti 19
C16.131.077.537 Loeysův-Dietzův syndrom 7
C16.131.077.550 Marfanův syndrom 105
C16.131.077.578 Moebiův syndrom 10
C16.131.077.592 moniletrichosis 1
C16.131.077.619 Nethertonův syndrom 2
C16.131.077.696 Cantrellova pentalogie 1
C16.131.077.703 POEMS syndrom 35
C16.131.077.730 Praderův-Williho syndrom 92
C16.131.077.735 nedostatek prolidázy
C16.131.077.740 Proteův syndrom 8
C16.131.077.745 prune belly syndrom 3
C16.131.077.790 zarděnky vrozené 20
C16.131.077.889 Sotosův syndrom 4
C16.131.077.919 Patauův syndrom 6
C16.131.077.929 syndrom trizomie 18 5
C16.131.077.938 Waardenburgův syndrom 10
C16.131.077.970 Zellwegerův syndrom 10
C16.131.240.400 vrozené srdeční vady 1 563
C16.131.240.400.021 syndrom delece 22q11 2
C16.131.240.400.021.500 DiGeorgeův syndrom 43
C16.131.240.400.044 Alagillův syndrom 19
C16.131.240.400.090 koarktace aorty 203
C16.131.240.400.118 aorto-ventrikulární tunel 1
C16.131.240.400.172 Barthův syndrom 6
C16.131.240.400.200 cor triatriatum 3
C16.131.240.400.210 anomálie koronárních cév 119
C16.131.240.400.220 crisscross srdce 1
C16.131.240.400.280 dextrokardie 21
C16.131.240.400.340 otevřená tepenná dučej 108
C16.131.240.400.395 Ebsteinova anomálie 46
C16.131.240.400.422 ectopia cordis 1
C16.131.240.400.450 Eisenmengerův syndrom 23
C16.131.240.400.560 defekty srdečního septa 164
C16.131.240.400.592 heterotaxe 7
C16.131.240.400.625 syndrom hypoplazie levého srdce 34
C16.131.240.400.701 levokardie 2
C16.131.240.400.715 syndrom dlouhého QT 218
C16.131.240.400.720 Marfanův syndrom 105
C16.131.240.400.784 Noonanové syndrom 52
C16.131.240.400.849 Fallotova tetralogie 163
C16.131.240.400.915 transpozice velkých cév 109
C16.131.240.400.920 trikuspidální atrézie 11
C16.131.240.400.960 Fallotova trilogie 2
C16.131.240.400.965 Patauův syndrom 6
C16.131.240.400.968 syndrom trizomie 18 5
C16.131.240.400.970 Turnerův syndrom 272
C16.131.240.400.975 jednokomorové srdce 3
C16.131.260.019 syndrom delece 22q11 2
C16.131.260.019.500 DiGeorgeův syndrom 43
C16.131.260.040 Angelmanův syndrom 35
C16.131.260.210 de Langeové syndrom 4
C16.131.260.260 Downův syndrom 510
C16.131.260.380 holoprosencefalie 9
C16.131.260.440 Jacobsenův syndrom 1
C16.131.260.700 Praderův-Williho syndrom 92
C16.131.260.905 Sotosův syndrom 4
C16.131.260.923 Patauův syndrom 6
C16.131.260.932 syndrom trizomie 18 5
C16.131.260.940 syndrom WAGR 3
C16.131.482.249 syndrom delece 22q11 2
C16.131.482.249.500 DiGeorgeův syndrom 43
C16.131.621.207.103 syndrom delece 22q11 2
C16.131.621.207.103.500 DiGeorgeův syndrom 43
C16.131.621.207.207 kleidokraniální dysplazie 7
C16.131.621.207.231 kraniofaciální dysostóza 28
C16.131.621.207.240 kraniosynostózy 56
C16.131.621.207.410 holoprosencefalie 9
C16.131.621.207.532 megalencefalie 7
C16.131.621.207.540 maxilofaciální abnormality 28
C16.131.621.207.620 mikrocefalie 78
C16.131.621.207.690 Noonanové syndrom 52
C16.131.621.207.700 orofaciodigitální syndromy 7
C16.131.621.207.707 plagiocefalie 3
C16.131.621.207.720 platybazie 12
C16.131.621.207.850 Rubinsteinův-Taybiho syndrom 12
C16.320.180.019 syndrom delece 22q11 2
C16.320.180.019.500 DiGeorgeův syndrom 43
C16.320.180.040 Angelmanův syndrom 35
C16.320.180.210 de Langeové syndrom 4
C16.320.180.260 Downův syndrom 510
C16.320.180.380 holoprosencefalie 9
C16.320.180.440 Jacobsenův syndrom 1
C16.320.180.700 Praderův-Williho syndrom 92
C16.320.180.905 Sotosův syndrom 4
C16.320.180.923 Patauův syndrom 6
C16.320.180.932 syndrom trizomie 18 5
C16.320.180.940 syndrom WAGR 3
C19.642.482 hypoparatyreóza 71
C19.642.482.500 syndrom delece 22q11 2
C19.642.482.500.500 DiGeorgeův syndrom 43